4.6 Article

MULTIFOCAL COMPLEX GLIONEURONAL TUMOR IN AN ELDERLY MAN: AN AUTOPSY STUDY: CASE REPORT

Journal

NEUROSURGERY
Volume 64, Issue 6, Pages 1193-1195

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1227/01.NEU.0000345640.40566.48

Keywords

Dysembryoplastic neuroepithelial tumor; Multicentricity; Pilocytic astrocytoma; Rosette-forming glioneuronal tumor; Third ventricle

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OBJECTIVE: The clinicopathological spectra of a dysembryoplastic neuroepithelial tumor (DNT) and a rosetteforming glioneuronal tumor (RGNT) are expanding. We report here the autopsy findings of a case of complex glioneuronal tumor with combined histological features of both a DNT and an RGNT. CLINICAL PRESENTATION: A 79-year-old man presented with a 1-month history of confusion and gait difficulties. A magnetic resonance imaging scan revealed obstructive hydrocephalus attributed to a mass in the posterior third ventricle. INTERVENTION: A third ventriculostomy was performed. Postoperatively, the mass remained unchanged in size for more than 14 months. Thirty-eight months after his initial manifestations, he experienced minor head trauma and was then hospitalized. Despite placement of an external for ventricular drain and other supportive treatment, he deteriorated and died. A full autopsy was performed, with emphasis on the brain. The mass lesion and a few independent microfoci situated primarily around the third ventricle showed histological features of pilocytic astrocytoma with recurrent hemorrhage. Far more numerous were microfoci with histological features of a DNT, including floating neurons, as well as typical RGNIT-associated, synaptophysin-positive rosettes and perivascular pseudo-rosettes. CONCLUSION: The advanced age of the patient, the coexisting histological features of the DNT and RGNT, and the distinctive anatomic distribution of the lesions, being centered on the third ventricle, may lend insight into the histogenetic relationship of a DNT, an RGNT, and mixed glioneuronal tumors.

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